Glucagonoma

Incidence

• Rare (1 in 20 million)
• Women 2-3x Men
• Larger c.f. other PNETs
  • 5-10cm in size
• Almost always in Pancreas
  • 65-75% body or tail
• Malignant in 50-80%
  • 80% of malignancies have liver mets at presentation
• Most sporadic, 5% MEN1

Presentation

• Classic presentation of “4 D’s”

  • Diabetes
  • Dermatitis
    • Necrolytic Migratory Erythema
  • DVT
  • Depression
    • Also get severe catabolic state with weight loss, fat and protein depletion, vitamin deficiencies

• Necrolytic Migratory Erythema

  • Characteristic skin lesion with glucagonoma syndrome
  • Seen in 2/3 patients, often before other symptoms
  • Thought to be due to severe amino acid deficiency
  • Can resolve with AA administration (IV)

Diagnosis

• Fasting glucagon level >1000pg/mL
• Normal < 100

Management

• Easily localised due to size and malignant behaviour
• Medical
  • Supplemental nutrition
  • Octreotide (to reverse catabolic state)
  • IV amino acids for dermatitis
  • DVT prophylaxis
• Surgery
  • Anatomic resection for resectable disease
• 5yr survival 85% if not Mets
• 5yr survival 60% with Mets